Abstract

OBJECTIVE The authors aimed to provide an updated and consolidated report on the epidemiology, management, and functional outcome of cases of myelomeningocele (MMC) in patients with scoliosis. METHODS A comprehensive literature search was performed using MEDLINE, Embase, Google Scholar, and the Cochrane Database of Systematic Reviews on cases of MMC in patients with scoliosis between 1980 and 2016. The initial search yielded 670 reports. After removing duplicates and applying inclusion criteria, we included 32 full-text original articles in this study. RESULTS Pooled statistical analysis of the included articles revealed the prevalence of scoliosis in MMC patients to be 53% (95% CI 0.42-0.64). Slightly more females (56%) are affected with both MMC and scoliosis than males. Motor level appears to be a significant predictor of prevalence, but not severity, of scoliosis in MMC patients. Treatment options for these patients include tethered cord release (TCR) and fusion surgeries. Curvature improvement and stabilization after TCR may be limited to patients with milder (< 50°) curves. Meanwhile, more aggressive fusion procedures such as a combined anterior-posterior approach may result in more favorable long-term scoliosis correction, albeit with greater complication rates. Quality of life metrics including ambulatory status and sitting stability are influenced by motor level of the lesion as well as the degree of the scoliosis curvature. CONCLUSIONS Scoliosis is among the most common and challenging comorbidities from which patients with MMC suffer. Although important epidemiological and management trends are evident, larger, prospective studies are needed to discover ways to more accurately counsel and more optimally treat these patients.

Abstract

PURPOSE OF REVIEW:

Obesity is a common comorbidity in individuals with spina bifida. Carrying excess weight exacerbates the inherent health challenges associated with spina bifida, impedes the individual's ability to self-manage their condition, and creates further challenges for family members and caregivers. This manuscript provides a narrative review of key issues for understanding and prevention of obesity in persons with spina bifida within the context of the social ecological model.

RECENT FINDINGS:

Specific variables related to obesity and spina bifida include individual factors (i.e., body composition and measurement issues, energy needs, eating patterns, physical activity, and sedentary activity) family factors (i.e., parenting/family, peers), community factors (i.e., culture, built environment, healthcare and healthcare providers, and school), and societal factors (i.e., policy issues). Due to the complex etiology of obesity and its increased prevalence in individuals with spina bifida, it is critical to initiate prevention efforts early with a multifactorial approach for this at-risk population. Increased research is warranted to support these efforts.

Abstract

OBJECTIVE:

Accelerated aging can occur in adult survivors of neurodevelopmental disorders, but has been narrowly studied in spina bifida myelomeningocele (SBM). Since discrete aspects of cognitive control and related neural network macrostructure deteriorate in normal aging, the specificity and trajectory of cognition and neuropathology incurred across adulthood in SBM were examined.

METHOD:

Adults (N = 120) with and without SBM completed working memory span and manipulation tasks, and an inhibitory control task. A subset (n = 53) underwent structural MRI. Effects of group, age, and their interaction on performance and select gray matter volumes were examined.

RESULTS:

Adults with SBM had significantly poorer working memory accuracy and overall inhibitory control performance than typical peers. Age negatively predicted inhibitory control. Group × Age significantly interacted on span accuracy; advanced age related to diminished performance in typical adults, but not in adults with SBM. SBM related to disproportionately enlarged cortical and putamen and reduced hippocampus volumes. Group × Age significantly interacted on cortical, but not subcortical gray matter volumes. Dorsolateral prefrontal, hippocampus, and putamen volumes negatively correlated with cognitive performance.

CONCLUSIONS:

Supporting previous literature, current findings elucidated a profile of executive impairment in SBM that was maintained in a parallel maturational trajectory to typical aging. Accelerated aging in cognitive control or subcortical gray matter was not supported in SBM. However, reductions in anterior and posterior cortical regions were exacerbated in older adults with SBM compared with typical peers. Overall results supported persistent anomalous neurodevelopmental maturation across the life span in SBM that related to diminished cognitive control. (PsycINFO Database Record

Abstract

PURPOSE OF REVIEW:

Spina bifida is caused by incomplete neural tube closure during the first trimester. This condition may lead to bowel and bladder dysfunction as well as truncal weakness and motor anomalies. Presentations vary between myelomeningoceles and lipomeningoceles and may result in different outcomes. This review seeks to explore our current understanding of the variations in outcomes between individuals with myelomeningocele and lipomeningocele.

RECENT FINDINGS:

Prenatal intervention has become a standard of care for prenatal diagnoses of myelomeningocele and has been shown to reduce shunt placement and improve motor skills. However, urological benefit from early intervention remains to be seen. Early surgical repair, however, may be beneficial for patients with lipomeningocele. Literature on the urological outcomes of patients with myelomeningocele and lipomeningocele is lacking. Further research is needed to better elucidate differences in long-term urological outcomes between these two pathologies.

Abstract

Attentional lapses are usually defined as temporary and often brief shifts of attention away from some primary task to unrelated internal information processing. This study addressed the incidence of attention lapses and differences in attentional functioning in 30 children with attention-deficit/hyperactivity disorder (ADHD), 26 healthy children, and 29 children with spina bifida myelomeningocele and hydrocephalus (SBH). Assessments were conducted using computerized tonic and phasic attention tests, the Symbol Digit Modalities Test (SDMT), and the Trail Making Test Form B (TMT-B). The group with SBH differed from normal controls on cognitive measures of attention and executive functions. The ADHD group obtained lower scores than the SBH group and healthy children. ANOVA results showed that there was an effect of shunt revisions and shunt-related infections on neuropsychological performance. Lapses of attention together with reaction time may thus represent important factors for the understanding of cognitive deficits in SBH.